Interstitial Lung Disease with Anti-melanoma Differentiation-associated Protein 5 Antibody: Rapidly Progressive Perilobular Opacity.

Interstitial Lung Disease with Anti-melanoma Differentiation-associated Protein 5 Antibody: Rapidly Progressive Perilobular Opacity.

Publication date: Jun 07, 2019

Objective Rapidly progressive interstitial lung disease (RP-ILD) with anti-melanoma differentiation-associated protein 5 (MDA5) antibody potentially presents with a fatal clinical course and requires early intensive treatment. Recently, perilobular opacity was reported to pathologically correspond to the acute phase of diffuse alveolar damage in RP-ILD with anti-MDA5 antibody. We aimed to investigate whether or not perilobular opacity was a common radiological finding in RP-ILD patients with anti-MDA5 antibody. Methods We conducted a retrospective review of the medical records of eight consecutive patients with RP-ILD with anti-MDA5 antibody. The clinical features and radiological findings of follow-up computed tomography (CT) during the course of their disease were evaluated. Results Among eight RP-ILD patients with anti-MDA-5 antibody, six showed perilobular opacity in the lower lobes, and the remaining two had only consolidation on high-resolution CT. Of note, the perilobular opacity in all six patients thickened and progressed to consolidation with a loss of lung volume in a short period. Despite intensive treatment, 6 patients (75%) died within 100 days after the first visit. Notably, the two patients with consolidation presented with a very rapid clinical course and died in 13 days each. In the two survivors, the perilobular opacity and consolidation recovered with improvement in the loss of lung volume. Conclusion Rapidly progressive perilobular opacity that thickens and progresses to consolidation is characteristic of RP-ILD with anti-MDA5 antibody. Chest physicians should immediately check the status of anti-MDA-5 antibody in order to initiate early aggressive therapy in RP-ILD patients with rapidly progressive perilobular opacity.

Chino, H., Sekine, A., Baba, T., Kitamura, H., Iwasawa, T., Okudela, K., Takemura, T., Itoh, H., Sato, S., Suzuki, Y., and Ogura, T. Interstitial Lung Disease with Anti-melanoma Differentiation-associated Protein 5 Antibody: Rapidly Progressive Perilobular Opacity. 22929. 2019 Intern Med.

Concepts Keywords
Antibody Dermatomyositis
Computed Tomography MDA5
Lung Branches of biology
Lung Volume Tomography
MDA Antibody
Melanoma Biochemistry
Opacity
Progressive

Semantics

Type Source Name
disease DOID amyopathic dermatomyositis
disease MESH amyopathic dermatomyositis
gene UNIPROT MAGEE1
gene UNIPROT IFIH1
pathway BSID Melanoma
disease DOID melanoma
disease MESH melanoma
disease DOID Interstitial Lung Disease
disease MESH Interstitial Lung Disease

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